Departments of 1Medical Imaging and Intervention and 2Pathology, Chang Gung ... University College of Medicine, 5 Fu Hsing Street, Kwei Shan Hsiang, ...
The British Journal of Radiology, 80 (2007), e212–e215
CASE REPORT
Radiation-induced skull base leiomyosarcoma presenting with intracerebral haemorrhage 1
C-H TOH,
MD,
1
H-F WONG,
MD,
2
S-M JUNG,
MD
and 1A-M WONG,
MD
Departments of 1Medical Imaging and Intervention and 2Pathology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, 5 Fu Hsing Street, Kwei Shan Hsiang, Tao-Yuan 333, Taiwan
ABSTRACT. Radiation-induced neoplasm including meningioma, glioma and sarcoma, is an uncommon but known consequence after therapeutic irradiation of pituitary tumour. Radiation-induced leiomyosarcoma due to previous sellar irradiation has only been reported once in the literature. We herein present the second case of radiationinduced leiomyosarcoma that happened 17 years after radiation therapy for pituitary adenoma. The case is unique in its initial presentation, i.e. acute intracerebral haemorrhage. To the best of our knowledge, such an acute and severe presentation has never been reported in radiation-induced neoplasm following radiotherapy for pituitary adenoma.
Pituitary adenomas are successfully treated with a range of treatment modalities, which include surgery, radiotherapy and medical treatment. Radiotherapy is principally employed to reduce the recurrence rate of incompletely excised non-secreting adenomas and in secreting pituitary tumours where hormonal control cannot be achieved with surgery and medical therapy. It is recognized that radiation is associated with the development of intracranial tumours after therapeutic cranial irradiation for acute lymphoblastic leukaemia [1], tinea capitis [2, 3] and intracranial tumours [4–6]. Ionizing radiation remains the only recognized environmental causative factor in the development of meningioma [7, 8]. A variety of tumours have been reported after cranial radiation therapy: meningiomas [3, 7–11], gliomas [5, 6, 10–13], sarcoma [11, 14–15], gliosarcoma [4], primitive neuroectodermal tumour [1, 11, 16] and ependymoma [9]. The fibrosarcoma is reported to be the most common radiation-induced intracranial sarcoma following radiation therapy for the pituitary adenomas [17]. Rarely, chondrosarcoma [18], osteosarcoma [19] and malignant fibrous histiocytoma [20] have also been reported. To the best of our knowledge, leiomyosarcoma after radiation therapy for pituitary adenomas has only been reported once [15]. In this article, we report a case of radiation-induced leiomyosarcoma that initially presented with acute intracerebral haemorrhage. To the best of our knowledge, such an acute and severe presentation has never been reported in radiationinduced neoplasm following surgery and radiotherapy for pituitary adenoma.
Address correspondence to: Dr Alex M Wong, 5 Fu Hsing Street, Kwei Shan Hsiang, Tao-Yuan, 333 Taiwan. E-mail: eldomtoh@ ms53.hinet.net
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Received 12 March 2006 Revised 8 May 2006 Accepted 15 June 2006 DOI: 10.1259/bjr/90471387 ’ 2007 The British Institute of Radiology
Case report A 40-year-old woman was admitted via the emergency department due to sudden onset of left-sided weakness and conscious disturbance. She had a pituitary macroadenoma about 17 years previously, and a tumour that had been surgically removed via the right frontotemporal approach. Postoperatively, she was also treated with radiation therapy because of the presence of residual tumour. The dose of radiotherapy was 60 Gy in 30 fractions over 7 weeks, and the treatment was delivered using a three-field technique with an anterior oblique and two lateral fields. A 6 cm diameter circular field was placed to encompass the preoperative extent of the macroadenoma, with a 1–2 cm margin. She had been well until this admission. Computed tomography (Figure 1a) showed a large intracerebral haematoma measuring about 6 cm in the right frontotemporal region, which caused subfalcine herniation. There was a soft tissue mass arising from and destroying the greater wing of the right sphenoid, extending into the orbital apex and middle cranial fossa (Figure 1b). Otherwise, there was an enlarged sella turcica with empty sella apppearance due to previous surgery. There was no definite residual tumour in the pituitary fossa and parasellar region. The patient underwent emergent craniotomy for decompression and an approximately 50 ml haematoma was removed. Angiography performed 1 week later (not shown) showed a hypervascular mass in the right temporal region with feeding arteries from the right middle meningeal and internal maxillary arteries. No vascular lesion such as arteriovenous malformation or dural arteriovenous fistula was found in the angiography. MR imaging was performed on a 1.5-T MR imaging system (Vision; Siemens, Erlangen, Germany). There was a 3.5 cm mass involving the right temporal bone and The British Journal of Radiology, September 2007
Case report: Radiation-induced skull base leiomyosarcoma with ICH
Figure 1. (a) Axial CT with contrast enhancement reveals a soft tissue mass arising from and destroying the greater wing of right sphenoid, extending into orbital apex and middle cranial fossa. A hyperdense hematoma is found in adjacent temporal lobe. The sellar is enlarged with empty sella appearance due to previous surgery. (b) Higher section shows extension of the hematoma to the right striatocapsular region and subfalcine herniation. There is evidence of previous craniotomy for pituitary macroadenoma with bony defect at right temporal bone.
sphenoid wing with both intradural and extradural components. The mass is hypointense on T1 weighted images and slightly hyperintense on T2 weighted images (4000/98 (repetition time msec/echo time msec)). On T1 weighted images (600/20) obtained after intravenous injection (Figure 2) of gadopentetate dimeglumine (Magnevist; Schering, Osaka, Japan), the mass enhanced moderately and heterogenously. A brain biopsy was performed and the frozen section studies showed malignant spindle cell tumour. So, a right fronto-temporal craniotomy was performed with removal of both intradural and extradural tumour components. The pathological studies (Figure 3) demonstrated proliferation of highly pleomorphic spindle cells with anaplastic nuclei, mitoses and necrosis. The immunohistochemical analysis demonstrated that tumour cells were diffusely positive for vimentin, focally positive for desmin, negative for S100, AE1/AE3 and EMA. The above pathological findings were suggestive of malignant tumour with smooth muscle differentiation. Thus, the diagnosis of leiomyosarcoma was confirmed. The patient died 1.5 months postoperatively as a result of pneumonia and respiratory failure.
Discussion The criteria of radiation-induced malignancies were originally described by Cahan et al [21], and were later The British Journal of Radiology, September 2007
revised by Murray et al [22] as follows: (1) the tumour arose in a previously irradiated field; (2) the new tumour is histologically different from the original condition; (3) there was no evidence of the new tumour at the time of radiation therapy; and (4) a latency period existed between irradiation and the development of the new tumour. In the present case, the secondary leiomyosarcoma fully satisfied the above-mentioned criteria of radiation-induced malignancies. Leiomyosarcomas are malignant mesenchymal neoplasms of smooth muscle differentiation that account for 5–10% of all soft tissue sarcomas. These tumours are often divided into three anatomic groups: soft tissue, cutaneous and vascular. The most common sites where they develop are the gastrointestinal tract, the urinary tract and the female genital tract. Radiation-induced leiomyosarcoma after radiation therapy for pituitary adenoma has only been reported once in the literature [15]. In that report, the patient presented with visual field defect 23 years after irradiation and the tumour was recognized in the left parasellar region. Our patient was the second case of radiation-induced leiomyosarcoma following pituitary surgery and radiation therapy. The leiomyosarcoma reported here was locally aggressive with both intradural and extradural components, and presented with acute intracerebral haemorrhage. The common presentation of radiation-induced tumour included mass effect, weakness, cranial nerve palsy, altered mental status and seizure, depending on the location of the tumour. e213
C-H Toh, H-F Wong, S-M Jung and A-M Wong
Figure 2. Contrast-enhanced T1 weighted MR image performed after surgical removal of right intracerebral hematoma demonstrating heterogenous enhancement of the mass. There is little residual hematoma in right temporal lobe.
Radiation-induced tumour with acute presentation of intracerebral haemorrhage, to the best of our knowledge, has never been reported in the literature. Recent researchers [11] have reported a cumulative risk of developing a second brain tumour in patients with pituitary adenoma treated with surgery and radiotherapy of 2.0% at 10 years and 2.4% at 20 years after
Figure 3. Photomicrography of a tissue sample showing proliferation of highly pleomorphic spindle cells with anaplastic nuclei, mitoses and necrosis. H&E. Original magnifications 6400. e214
treatment, with a relative risk compared with the normal population of 10.5. Tumours were diagnosed 6–34 years after radiotherapy, with a mean of 6.7 years for astrocytoma and 20.8 years for meningeal tumours. Radiation-induced neoplasm with latency as short as 1 year had also been reported [12]. Most cases were identified within the region of entry of radiation fields commonly in the temporal region [6], and less commonly within the pituitary fossa. Age, sex, pituitary adenoma type, radiation dose, tumour extent and medical treatment were not found to be predictive of the development of second tumours on statistically analysis. The likelihood of developing a secondary tumour in an intracranial region previously irradiated for therapeutic purposes is relatively rare compared with other longterm complications of radiotherapy, such as recurrence of the primary lesion and necrosis of the irradiated tissue [4]. However, because of long life expectancy in patients with pituitary adenoma the possible risk of a radiationinduced tumour should be carefully evaluated. Other factors, such as the patient’s age at onset of the adenoma, tumour size, the extent of surgical removal, the use of medical treatment, the residual reserve of the gland and the feasibility of monitoring of the patient, should be taken into account before performing radiotherapy in these patients. In patients with new or recurring symptoms after treatment with standard radiation therapy, secondary tumour development must be considered in the differential diagnosis. It is therefore imperative that clinicians managing patients with pituitary tumour be aware of the long-term complications of radiation therapy. Although there is no evidence demonstrating decreased incidence of radiation-induced tumour in more conformal radiation treatment techniques, no cases of second tumour after fractionated stereotatic radiotherapy or radiosurgery for pituitary adenomas have been reported to date. Taking this into consideration, it does emphasize the need for more conformal radiation treatment techniques for benign tumours. The use of these conformal radiotherapy procedures not only prevents further tumour regrowth, but also associates with decreased incidence of hormomal insufficiency and optic neuropathy compared with conventional radiotherapy [6]. Therefore, treatment of sellar tumours with conventional fractionated radiotherapy should be carefully considered and perhaps used primarily in those cases for which radiosurgery is not appropriate. In spite of this, the contribution of radiation-induced tumour to long-term mortality is considered minimal when compared with risk of mortality from cerebrovascular events [23–24]. Therefore, the low incidence of second brain tumours should therefore not preclude the use of radiotherapy as an effective treatment modality in patients with uncontrolled pituitary adenomas. In conclusion, we have reported the second case of radiation-induced leiomyosarcoma and the first case of radiation-induced neoplasm presented with severe intracranial haemorrhage following surgical and radiation therapy of pituitary adenoma. It is therefore imperative that clinicians managing patients with pituitary tumour be aware of the long-term complication of radiation therapy. The British Journal of Radiology, September 2007
Case report: Radiation-induced skull base leiomyosarcoma with ICH
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